Nephrotic syndrome in children

There is something wrong with your child's kidneys. There is a suspicion of nephrotic syndrome? You may be uncertain about what to expect for you and your child. This information sheet gives you a first overview of the rare clinical picture.

At a glance

In idiopathic nephrotic syndrome (iNS), the affected child loses proteins through the urine. Water retention in the body (edema) occurs. After a few years, the disease heals in most children. Only rarely does a permanent restriction of kidney function develop.

An effective treatment is medication that suppresses the body's defense system. Recidivism is common. The goal of treatment is then to reduce relapses while keeping the side effects of the medication as low as possible.

The disease

The kidneys work like a sieve or filter. They filter out pollutants. Retain valuable substances for the body. In nephrotic syndrome, the sieve is leaking. Valuable substances such as proteins end up in the urine. Thus proteins are missing in the blood. Since the proteins in the blood normally hold the water, more water now gets into other ties. These water retention symptoms can show up as swelling. The technical term is edema.

Around 250 children in Germany are diagnosed with idiopathic nephrotic syndrome (iNS) every year. Mostly children between 1 and 10 years are affected. Idiopathic means that the cause is unclear. However, it is known that the body's own defense system plays a role in this process.

Signs and complaints

Typical signs are:

edema, especially in the face (eyelids), legs and feet, and genital area (scrotum or labia)

dark discolored and foamy urine

too few proteins (proteins) in the blood

Increased fat levels in the blood

Other complaints may occur, such as nausea, fatigue or high blood prere. In addition, children are susceptible to infections because they lack proteins that otherwise ward off pathogens. Vessels can clog more easily due to the loss of water in the blood. The risk of blood clots (thromboses) is increased.

If left untreated, the kidneys can be so severely damaged that they fail completely (kidney failure). Fluid accumulation can also become threatening if left untreated, for example in the lungs.

The iNS occurs in spurts. This means that there are times without symptoms and times when signs of the disease appear. The typical iNS usually disappears when the affected person becomes an adult.

Investigations

The doctor will interview you and your child in detail and perform a physical exam. Your child's urine and blood are tested in the laboratory and the kidneys are examined by ultrasound.

The doctor will also check whether your child has iNS or whether there is a specific cause of nephrotic syndrome, for example, a hereditary disease or inflammation of the blood vessels, as a result of which nephrotic syndrome can develop.

Experts recommend removal of tie from the kidney only in certain situations, for example, when medications do not work or when the child is older than 10 years at the onset of the disease.

The treatment

If iNS occurs for the first time, experts recommend medications that act like cortisone. In technical language they are called glucocorticoids. These suppress the body's own defense system. Relapses of disease and permanent kidney damage are thus avoided. In about 9 out of 10 affected children, these cortisone-like drugs are effective.

If the disease occurs for the first time, experts currently recommend taking prednisone, an active ingredient similar to cortisone, for a period of 12 weeks. Studies indicate that relapses are less frequent after 12 weeks of treatment than after 8 weeks. Whether an even longer treatment period can prevent more relapses is unclear according to current studies.

According to experts, affected children should take the prednisone once a day in the morning for the first 6 weeks. For another 6 weeks, the medication is used only every other day. The dose depends on the child's size and weight.

Despite treatment, a relapse occurs in about 3 out of 4 affected children. Experts speak of a relapse. Repeated relapses occur in many. Then taking the prednisone for a shorter period of time than the first time. Usually the drug is again well effective, so that the kidneys recover again. The goal is to keep medication side effects low, such as stunted growth, bone loss, weight gain, lens clouding of the eyes, or high blood prere.

Things get difficult when the prednisone doesn't work or stops working. Affected people often need repeated or permanent use of other medications that suppress the body's defenses (immunosuppressants).

Very rarely, the kidneys may stop working permanently. Kidney weakness (renal insufficiency) develops. Then treatments are needed that take over the work of the kidneys – in technical language they are called: Kidney Replacement Therapies. This includes blood washing (dialysis). The kidney transplant.

Close cooperation between parents and a team of pediatricians specializing in kidney diseases (pediatric nephrology) can usually prevent permanent consequences of the disease and damage caused by the medication. Regular check-ups in a nephrology practice or outpatient clinic are also recommended in adulthood.

What you can do yourself

Your child does not need to take it easy physically, he can do everything like other children. Even during a relapse, your child does not need bed rest.

During an episode, your child should be on a low-salt diet. It is best to discuss the amount of drinking with the medical team.

To detect a relapse in time, check your child's urine for protein with a test strip in the morning.

Vaccinations protect against certain infections. However, they can trigger a relapse of iNS. Get advice on this subject.

With support, everyday life can be managed more easily. If necessary, you as a family can receive psychosocial support.

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